hemoglobinuria

hemoglobinuria

the presence of free hemoglobin in the urine. adj., adj hemoglobinu´ric.

march hemoglobinuria hemolysis caused by repeated uncushioned shocks or trauma to some body part, such as in some soldiers on long marches, in marathon runners, and in karate practitioners.

paroxysmal cold hemoglobinuria an autoimmune or postviral disease in which there is a biphasic IgG antibody directed against the P blood group antigen. It is marked by episodes of hemoglobinemia and hemoglobinuria after exposure to cold and is detected by the Donath-Landsteiner test. The condition is treated with prednisone and cyclophosphamide and by protection from exposure to cold.

paroxysmal nocturnal hemoglobinuria (PNH) an acquired blood cell abnormality with proliferation of abnormal red blood cells (PNH cells) that are readily hemolyzed by complement, and episodes of severe hemolysis and thrombosis, particularly of the hepatic veins. It is detected by the ham test. Treatment is with androgens or prednisone and, during thrombotic episodes, with heparin.

he·mo·glo·bi·nu·ri·a

(hē'mō-glō'bi-nyū'rē-ă),

The presence of hemoglobin in the urine, including certain closely related pigments that are formed from slight alteration of the hemoglobin molecule; when present in sufficient quantities, they result in the urine, being colored in shades varying from light reddish-yellow to fairly dark red.

[hemoglobin + G. ouron, urine]

hemoglobinuria

paroxysmal cold hemoglobinuria an autoimmune or postviral disease marked by episodes of hemoglobinemia and hemoglobinuria after exposure to cold, caused by complement-dependent hemolysis due to Donath-Landsteiner antibody.

hemoglobinuria

(hē′mə-glō′bə-no͝o′ē-ə, -nyo͝or′-)

n.

The presence of hemoglobin in the urine.

he′mo·glo′bi·nu′ric adj.

hemoglobinuria

[-oo͡r′ē·ə]

Etymology: Gk, haima + L, globus, ball; Gk, ouron, urine

abnormal presence in the urine of hemoglobin that is not attached to red blood cells. Hemoglobinuria can result from various autoimmune diseases or episodic hemolytic disorders. It can be diagnosed by using a dipstick reagent that is sensitive to free hemoglobin. Kinds of hemoglobinuria include cold hemoglobinuria, march hemoglobinuria, and nocturnal hemoglobinuria. Also spelled haemoglobinuria. Also called toxic hemoglobinuria. -hemoglobinuric,adj.

he·mo·glo·bi·nu·ri·a

(hē'mō-glō'bi-nyūr'ē-ă)

The presence of hemoglobin in the urine, including certain closely related pigments formed from slight alteration of the hemoglobin molecule; indicative of intravascular hemolysis or of bleeding into the urinary tract, with hemolysis there. The urine may be reddish-yellow to dark red. Synonym(s): haemoglobinuria.

[hemoglobin + G. ouron, urine]

he·mo·glo·bi·nu·ri·a

(hē'mō-glō'bi-nyūr'ē-ă)

Presence of hemoglobin in urine; when present in sufficient quantities, they result in the urine's being colored in shades varying from light reddish-yellow to fairly dark red. Synonym(s): haemoglobinuria.

[hemoglobin + G. ouron, urine]

hemoglobinuria

the presence of free hemoglobin in the urine. In true hemoglobinuria the protein has originated from erythrocytes hemolyzed within the vascular system. In false hemoglobinuria it comes from erythrocytes in the urine that have hemolyzed there, leaving red cell envelopes in the urinary sediment. In both forms the urine is colored dark red to almost black.

bacillary hemoglobinuria

is an acute, highly fatal toxemia of cattle and sheep caused by Clostridium haemolyticum (C. novyi type D). It is characterized by hemoglobinuria, jaundice and sudden death with large, usually single infarcts in the liver.

cold hemoglobinuria

intravascular hemolysis occurs in the blood vessels in the intestinal wall immediately after the calf, rarely an adult, has drunk a large amount of very cold water after a period of deprivation. Transient hemoglobinuria may occur. See also cold anemia.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired clonal hematopoietic stem cell disorder, which is caused by a somatic mutation in the phosphatidylinositol glycan-complementation class A gene, with an estimated prevalence of 1-2 cases per million people in the United States (1-2).

The data were presented at the 51st Annual Meeting of the American Society of Hematology (ASH) in a poster titled "Chronic Renal Insufficiency in Japanese Patients with Paroxysmal Nocturnal Hemoglobinuria (PNH): Improvement with Eculizumab Treatment in the Long-Term Follow-up of the AEGIS Study.

has announced that the Committee for Human Medicinal Products (CHMP) of the European Medicines Agency (EMEA) adopted a positive opinion recommending marketing authorization for Soliris (eculizumab) for the treatment of patients with paroxysmal nocturnal hemoglobinuria (PNH).

Food and Drug Administration for its lead product candidate Soliris (eculizumab) as a treatment for patients diagnosed with paroxysmal nocturnal hemoglobinuria (PNH), a rare life-threatening genetic blood disorder.

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